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Abstract: Sickle cell disease (SCD) is a collection of inherited blood disorders that affect a substantial number of people in the U.S., particularly African Americans. People with SCD have an abnormal type of hemoglobin, Hb S, which polymerizes when deoxygenated, causing the red blood cells to become misshapen and rigid. Individuals with SCD are at higher risk of morbidity and mortality from infections, vaso-occlusive pain crises, acute chest syndrome, and other complications. Addressing the public health needs related to SCD is an important step toward improving outcomes and maintaining health for those affected by the disorder. The objective of this study was to review public health activities focusing on SCD and define the need to address it more comprehensively from a public health perspective. We found that there has been some progress in the development of SCD-related public health activities. Such activities include establishing newborn screening (NBS) for SCD with all states currently having universal NBS programs. However, additional areas needing focus include strengthening surveillance and monitoring of disease occurrence and health outcomes, enhancing adherence to health maintenance guidelines, increasing knowledge and awareness among those affected, and improving healthcare access and utilization. These and other activities discussed in this paper can help strengthen public health efforts to address SCD. [Copyright &y& Elsevier]

Background: Patients with sickle cell disease (SCD) often use emergency department services to obtain medical care. Limited information is available about emergency department use among patients with SCD. Purpose: This study assessed characteristics of emergency department visits made nationally by patients with SCD. Methods: Data from the National Hospital Ambulatory Medical Care Survey (NHAMCS) for the years 1999–2007 were analyzed. The NHAMCS is a survey of hospital emergency department and outpatient visits. Emergency department visits by patients with SCD were identified using ICD-9-CM codes, and nationally weighted estimates were calculated. Results: On average, approximately 197,333 emergency department visits were estimated to have occurred each year between 1999 and 2007 with SCD as one of the diagnoses listed. The expected source of payment was private insurance for 14%, Medicaid/State Children''s Health Insurance Program for 58%, Medicare for 14%, and other/unknown for 15%. Approximately 29% of visits resulted in hospital admission; this was 37% among patients aged 0–19 years, and 26% among patients aged ≥20 years. The episode of care was indicated as a follow-up visit for 23% of the visits. Patient-cited reasons for the emergency department visit included chest pain (11%); other pain or unspecified pain (67%); fever/infection (6%); and shortness of breath/breathing problem/cough (5%), among other reasons. Conclusions: Substantial numbers of emergency department visits occur among people with SCD. The most common reason for the emergency department visits is pain symptoms. The findings of this study can help to improve health services delivery and utilization among patients with SCD. [Copyright &y& Elsevier]